Everolimus for Treatment of Pseudomyogenic Hemangioendothelioma

Pseudomyogenic hemangioendothelioma (PMH) is a recently described vascular neoplasm that occurs most commonly in the soft tissue of the distal extremities of young adults. Metastatic PMH can be fatal and there are no effective medications. We describe a case of a 15-year-old boy with metastatic PMH,...

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Bibliographic Details
Published inJournal of pediatric hematology/oncology Vol. 39; no. 6; p. e328
Main Authors Ozeki, Michio, Nozawa, Akifumi, Kanda, Kaori, Hori, Tomohiro, Nagano, Akihito, Shimada, Akira, Miyazaki, Tatsuhiko, Fukao, Toshiyuki
Format Journal Article
LanguageEnglish
Published United States 01.08.2017
Subjects
Adolescent
Everolimus - pharmacology
Everolimus - therapeutic use
Hemangioendothelioma - drug therapy
Hemangioendothelioma - pathology
Humans
Immunohistochemistry
Male
Neoplasm Metastasis
TOR Serine-Threonine Kinases - analysis
TOR Serine-Threonine Kinases - antagonists & inhibitors
Tumor Burden - drug effects
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Summary:Pseudomyogenic hemangioendothelioma (PMH) is a recently described vascular neoplasm that occurs most commonly in the soft tissue of the distal extremities of young adults. Metastatic PMH can be fatal and there are no effective medications. We describe a case of a 15-year-old boy with metastatic PMH, who responded to treatment with everolimus, a mammalian target of rapamycin inhibitor. Immunohistochemistry showed that mammalian target of rapamycin was expressed in PMH biopsy specimens, which may explain the reduction in PMH tumor size following treatment.
ISSN:1536-3678
DOI:10.1097/mph.0000000000000778